Suppr超能文献

生长激素(GH)治疗特纳综合征患者的最终身高:高剂量单独使用GH及晚期雌激素治疗的指征。意大利特纳综合征研究组

Final height of patients with Turner's syndrome treated with growth hormone (GH): indications for GH therapy alone at high doses and late estrogen therapy. Italian Study Group for Turner Syndrome.

作者信息

Cacciari E, Mazzanti L

机构信息

Department of Pediatrics, University of Bologna, Italy.

出版信息

J Clin Endocrinol Metab. 1999 Dec;84(12):4510-5. doi: 10.1210/jcem.84.12.6175.

Abstract

We report final height data of patients with Turner's syndrome collected by the Italian Study Group for Turner's Syndrome. One hundred and thirty-five patients reached their final height during GH therapy with different therapeutic regimens (dose and combination). They were divided into 3 groups: group A, 74 patients with high doses of GH (1 IU/kg/week) for at least 2 yr; group A1, GH alone and estrogen therapy added not before 14 yr of chronological age (47 patients, of whom 30 were treated for >4 yr and 10 for >6 yr); group A2, GH plus ethinyl estradiol (17 patients) or GH plus oxandrolone (10 patients); group B, 51 patients with low doses of GH (0.5 IU/kg-week) and high doses of GH for less than 2 yr; and group C, 10 patients with high doses of GH with spontaneous menarche. In contrast to the patients of groups B and C, the patients of group A showed a significantly higher final height (mean, 147.5+/-6.5 cm) than their projected height (mean, 142.9+/-6.4 cm). They showed also a significantly higher final height compared to the subjects of groups B (mean, 145.6+/-5.7 cm) and C (mean, 143.0+/-5.3). Among the patients of group A, the best results were obtained in the patients of group A1 treated with GH alone at high doses and for a longer period (4 yr, 149.3+/-6.4 cm; 6 yr, 153.8+/-4.0 cm). Karyotype, GH secretion, and birth weight did not influence the efficacy of GH therapy. A low target height and a high prevalence of a spontaneous ovarian activity or menarche may negatively influence the effect of GH therapy. Estrogens did not improve final height when added to GH therapy. The use of small doses of oxandrolone was not effective in our experience. GH therapy provides a satisfactory auxological result, especially with high doses of GH alone, given for a long period of time. Optimization of the treatment would seem to require the identification of the ideal age for starting therapy, and this is only possible with a specially designed multicenter study.

摘要

我们报告了意大利特纳综合征研究小组收集的特纳综合征患者的最终身高数据。135例患者在接受生长激素(GH)治疗且治疗方案(剂量和联合用药)不同的过程中达到了最终身高。他们被分为3组:A组,74例接受高剂量GH(1 IU/kg/周)治疗至少2年的患者;A1组,仅接受GH治疗且在实足年龄14岁之前未添加雌激素治疗(47例患者,其中30例接受治疗超过4年,10例超过6年);A2组,GH加乙炔雌二醇(17例患者)或GH加氧雄龙(10例患者);B组,51例接受低剂量GH(0.5 IU/kg-周)且高剂量GH治疗少于2年的患者;C组,10例接受高剂量GH且有自然月经初潮的患者。与B组和C组患者相比,A组患者的最终身高(平均147.5±6.5 cm)显著高于其预测身高(平均142.9±6.4 cm)。与B组(平均145.6±5.7 cm)和C组(平均143.0±5.3 cm)的受试者相比,他们的最终身高也显著更高。在A组患者中,A1组接受高剂量且较长时间单独GH治疗的患者取得了最佳结果(4年,149.3±6.4 cm;6年,153.8±4.0 cm)。核型、GH分泌和出生体重不影响GH治疗的疗效。低目标身高以及自然卵巢活动或月经初潮的高发生率可能对GH治疗效果产生负面影响。在GH治疗中添加雌激素并不能提高最终身高。根据我们的经验,使用小剂量氧雄龙无效。GH治疗能提供令人满意的体格学结果,尤其是长时间单独使用高剂量GH时。治疗的优化似乎需要确定开始治疗的理想年龄,而这只有通过专门设计的多中心研究才有可能实现。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验